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RICHTER'S SYNDROME IN PATIENTS WITH CHRONIC LYMPHOCYTIC LEUKEMIA

Year 1999, Volume: 12 Issue: 4, 183 - 185, 03.12.2016

Abstract

Objective: The development of a higher grade lymphoma in patients with chronic lymphocytic leukemia (CLL) is defined as Richter's syndrome (RS). The aim of this study was to analyze all CLL patients seen at Marmara University Hospital during 7 years of follow up in order to determine the incidence, clinical features and treatment outcome of patients who transformed to RS.
Patients and Method: Fifty four patients with CLL were evaluated in this retrospective study, where records of all patients with CLL, who are followed up at the haematology out-patient clinics during 7 years, were examined in order to determine whether Richter transformation had occurred at any time during the course of the disease.
Results: Richter's syndrome was observed in 3 out of 54 patient, giving an incidence of 5.6%. The presenting features of RS have been systemic symptoms, rapidly progressive lymphadenopathy, and elevation of lactate dehydrogenase LDH. None of the patients responded to systemic chemotherapy and the median survival was only 4 months following the diagnosis of RS.
Conclusion: Early diagnosis of RS is possible with the recognition of the warning symptoms and signs, but treatment remains unsatisfactory. The study highlights the need to develop new strategies for the successful treatment of RS.
Key Words: Richter's syndrome, Chronic
Lymphocytic Leukemia

References

  • /. Richter MR. Generalized reticular cell sarcoma of lymph nodes associated with lymphatic leukemia. Am J Pathol 1928;4:285-292.
  • Trump DL, Mann RB, Phelps R, Roberts ft Conley
  • CL. Richters Syndrome: Diffuse histiocytic
  • lymphoma in patients with chronic lymphocytic leukemia. A report of five cases and review of the literature. Am J Med 1980:68:539-548.
  • Robertson LE, Pugh W, O'Brien S, et al. Richters syndrome: A report on 39 patients. J Clin Oncol 1993; 11:1985-1989.
  • Wetzler M, Rurzrock R, Goodacre AM, McLaughlin P, Ku 5, Talpaz M. Transformation of chronic lymphocytic leukemia to lymphoma of true histiocytic type. Cancer 1995;76:609-617.
  • Williams J, Schned A, Cotelingam JD, Jaffe ES. Chronic lymphocytic leukemia with coexistent Hodgkin s disease. Implications for the origin of the Reed-Sternberg cell. Am J Surg Pathol 1991 ; 15:33- 42.
  • Poucar K, Rydell RE. Richters syndrome in chronic lymphocytic leukemia. Cancer I980;46-1 18-134.
  • Cheson BD, Bennett JM, Grever M, et al. national Cancer Institute-sponsored working group guidelines for chronic lymphocytic leukemia: revised guidelines for diagnosis and treatment. Blood 1996;87:4990-4997.
  • Rai KR, Sawitsky A, Cronkite EP, Chanana AD, Levy RM, Pasternack BS. Clinical staging of chronic lymphocytic leukemia. Blood 1995:46:219-234.
  • Rozman C, Montserrat E. Chronic lymphocytic leukemia. M Engl J Med 1995:333:1052-1057.
  • O'Brien S, del Giglio A, Keating M. Advances in the biology and treatment of B-cell chronic lymphocytic leukemia. Blood 1995;85:307-318.
  • Matolcsy A, Inghirami G, Knowles DM. Molecular genetic demonstration of the diverse evolution of Richter's syndrome (chronic lymphocytic leukemia and subsequent large cell lymphoma). Blood 1994;83:1363-1372.
  • McDonnell JM, Beschorner WE, Stall SP, Spivak JL, Mann RB. Richters syndrome with two different B- cell clones. Cancer 1986;58:2031-2037.
  • fleslop HE, Fitzgerald PH, Beard MEJ. Sustained complete remission in Richters syndrome. Cancer 1987;59:1036-1039.
  • Gordon LI, Harrington D, Andersen J, et al. Comparison of a second-generation combination chemotherapeutic regimen (m-BACOD) with a standard regimen (CHOP) for advanced non- Hodgkin's lymphoma. H Engl J Med ¡992:327: 1342-1349.
Year 1999, Volume: 12 Issue: 4, 183 - 185, 03.12.2016

Abstract

References

  • /. Richter MR. Generalized reticular cell sarcoma of lymph nodes associated with lymphatic leukemia. Am J Pathol 1928;4:285-292.
  • Trump DL, Mann RB, Phelps R, Roberts ft Conley
  • CL. Richters Syndrome: Diffuse histiocytic
  • lymphoma in patients with chronic lymphocytic leukemia. A report of five cases and review of the literature. Am J Med 1980:68:539-548.
  • Robertson LE, Pugh W, O'Brien S, et al. Richters syndrome: A report on 39 patients. J Clin Oncol 1993; 11:1985-1989.
  • Wetzler M, Rurzrock R, Goodacre AM, McLaughlin P, Ku 5, Talpaz M. Transformation of chronic lymphocytic leukemia to lymphoma of true histiocytic type. Cancer 1995;76:609-617.
  • Williams J, Schned A, Cotelingam JD, Jaffe ES. Chronic lymphocytic leukemia with coexistent Hodgkin s disease. Implications for the origin of the Reed-Sternberg cell. Am J Surg Pathol 1991 ; 15:33- 42.
  • Poucar K, Rydell RE. Richters syndrome in chronic lymphocytic leukemia. Cancer I980;46-1 18-134.
  • Cheson BD, Bennett JM, Grever M, et al. national Cancer Institute-sponsored working group guidelines for chronic lymphocytic leukemia: revised guidelines for diagnosis and treatment. Blood 1996;87:4990-4997.
  • Rai KR, Sawitsky A, Cronkite EP, Chanana AD, Levy RM, Pasternack BS. Clinical staging of chronic lymphocytic leukemia. Blood 1995:46:219-234.
  • Rozman C, Montserrat E. Chronic lymphocytic leukemia. M Engl J Med 1995:333:1052-1057.
  • O'Brien S, del Giglio A, Keating M. Advances in the biology and treatment of B-cell chronic lymphocytic leukemia. Blood 1995;85:307-318.
  • Matolcsy A, Inghirami G, Knowles DM. Molecular genetic demonstration of the diverse evolution of Richter's syndrome (chronic lymphocytic leukemia and subsequent large cell lymphoma). Blood 1994;83:1363-1372.
  • McDonnell JM, Beschorner WE, Stall SP, Spivak JL, Mann RB. Richters syndrome with two different B- cell clones. Cancer 1986;58:2031-2037.
  • fleslop HE, Fitzgerald PH, Beard MEJ. Sustained complete remission in Richters syndrome. Cancer 1987;59:1036-1039.
  • Gordon LI, Harrington D, Andersen J, et al. Comparison of a second-generation combination chemotherapeutic regimen (m-BACOD) with a standard regimen (CHOP) for advanced non- Hodgkin's lymphoma. H Engl J Med ¡992:327: 1342-1349.
There are 16 citations in total.

Details

Journal Section Original Research
Authors

Ahmet Toprak This is me

Siret Ratip This is me

Tülin Budak Alpdoğan This is me

Önder Alpdoğan This is me

Sevgi Küllü This is me

Mahmut Bayık This is me

Publication Date December 3, 2016
Published in Issue Year 1999 Volume: 12 Issue: 4

Cite

APA Toprak, A., Ratip, S., Budak Alpdoğan, T., Alpdoğan, Ö., et al. (2016). RICHTER’S SYNDROME IN PATIENTS WITH CHRONIC LYMPHOCYTIC LEUKEMIA. Marmara Medical Journal, 12(4), 183-185.
AMA Toprak A, Ratip S, Budak Alpdoğan T, Alpdoğan Ö, Küllü S, Bayık M. RICHTER’S SYNDROME IN PATIENTS WITH CHRONIC LYMPHOCYTIC LEUKEMIA. Marmara Med J. June 2016;12(4):183-185.
Chicago Toprak, Ahmet, Siret Ratip, Tülin Budak Alpdoğan, Önder Alpdoğan, Sevgi Küllü, and Mahmut Bayık. “RICHTER’S SYNDROME IN PATIENTS WITH CHRONIC LYMPHOCYTIC LEUKEMIA”. Marmara Medical Journal 12, no. 4 (June 2016): 183-85.
EndNote Toprak A, Ratip S, Budak Alpdoğan T, Alpdoğan Ö, Küllü S, Bayık M (June 1, 2016) RICHTER’S SYNDROME IN PATIENTS WITH CHRONIC LYMPHOCYTIC LEUKEMIA. Marmara Medical Journal 12 4 183–185.
IEEE A. Toprak, S. Ratip, T. Budak Alpdoğan, Ö. Alpdoğan, S. Küllü, and M. Bayık, “RICHTER’S SYNDROME IN PATIENTS WITH CHRONIC LYMPHOCYTIC LEUKEMIA”, Marmara Med J, vol. 12, no. 4, pp. 183–185, 2016.
ISNAD Toprak, Ahmet et al. “RICHTER’S SYNDROME IN PATIENTS WITH CHRONIC LYMPHOCYTIC LEUKEMIA”. Marmara Medical Journal 12/4 (June 2016), 183-185.
JAMA Toprak A, Ratip S, Budak Alpdoğan T, Alpdoğan Ö, Küllü S, Bayık M. RICHTER’S SYNDROME IN PATIENTS WITH CHRONIC LYMPHOCYTIC LEUKEMIA. Marmara Med J. 2016;12:183–185.
MLA Toprak, Ahmet et al. “RICHTER’S SYNDROME IN PATIENTS WITH CHRONIC LYMPHOCYTIC LEUKEMIA”. Marmara Medical Journal, vol. 12, no. 4, 2016, pp. 183-5.
Vancouver Toprak A, Ratip S, Budak Alpdoğan T, Alpdoğan Ö, Küllü S, Bayık M. RICHTER’S SYNDROME IN PATIENTS WITH CHRONIC LYMPHOCYTIC LEUKEMIA. Marmara Med J. 2016;12(4):183-5.